Enfermedad de Creutzfeldt-Jakob: experiencia de 5 años en un hospital terciario de Chile / Creutzfeldt-Jakob disease, experience in 17 patients

Background: Creutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations. Aim: To characterize clinical, imaging and electroencephalography findings in patients with a probable CJD. Patients and Methods: A case series study of patients admitted in the Neurology department at a public hospital, between 2014 and 2019. Demographic, clinical, imaging, and electroencephalographic data of patients with probable CJD were analyzed. Results: Seventeen patients aged 63 ± 11 years (53% women) with a probable CJD were gathered. The incidence was 4.7 cases/year per million inhabitants. Twenty four percent of patients had a family history of CJD. The median time between the onset of symptoms and the hospital admission was three months with a survival of four months. The most common clinical manifestations were an amnesic syndrome in 88%, myoclonus in 76%, frontal syndrome and ataxia in 71%. Brain MRI was abnormal in all patients. The preponderant finding was the involvement of the caudate nucleus in 82% of cases. In the EEG, 94% of patients had abnormalities. All had a theta-delta slowing as a base rhythm. The pseudo-periodic pattern was observed in the 29% and status epilepticus in 18%. Conclusions: In this group of patients we observed the heterogeneity of the clinical manifestations of the disease, the frequent imaging and electroencephalographic alterations and the short evolution time leading to death.

[Creutzfeldt-Jakob disease, experience in 17 patients]. / Enfermedad de Creutzfeldt-Jakob: experiencia de 5 años en un hospital terciario de Chile.

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations. AIM: To characterize clinical, imaging and electroencephalography findings in patients with a probable CJD. PATIENTS AND METHODS: A case series study of patients admitted in the Neurology department at a public hospital, between 2014 and 2019. Demographic, clinical, imaging, and electroencephalographic data of patients with probable CJD were analyzed. RESULTS: Seventeen patients aged 63 ± 11 years (53% women) with a probable CJD were gathered. The incidence was 4.7 cases/year per million inhabitants. Twenty four percent of patients had a family history of CJD. The median time between the onset of symptoms and the hospital admission was three months with a survival of four months. The most common clinical manifestations were an amnesic syndrome in 88%, myoclonus in 76%, frontal syndrome and ataxia in 71%. Brain MRI was abnormal in all patients. The preponderant finding was the involvement of the caudate nucleus in 82% of cases. In the EEG, 94% of patients had abnormalities. All had a theta-delta slowing as a base rhythm. The pseudo-periodic pattern was observed in the 29% and status epilepticus in 18%. CONCLUSIONS: In this group of patients we observed the heterogeneity of the clinical manifestations of the disease, the frequent imaging and electroencephalographic alterations and the short evolution time leading to death.

Afasia como debut de estado hiperosmolar hiperglicémico. Caso clínico / Aphasia as the presenting symptom of a hyperglycemic hyperosmolar state. Case report

Neurological manifestations such as seizures, disorders of consciousness and abnormal movements such as hemichorea and hemiballismus can be the presenting symptoms of hyperglycemic hyperosmolar states. Exceptionally, focal signs as hemiparesis or aphasia are described. We report a 66-year-old man, presenting with nonfluent aphasia and right subtle hemiparesis. The computed tomography, computed tomography angiography and brain magnetic resonance did not show acute ischemic lesions or obstruction of arterial vessels. The initial laboratory evaluation disclosed a blood glucose of 936 mg/dL, a plasma osmolality of 331 mOsm/Kg, and positive plasma ketones. After the treatment of hyperglycemia and hyperosmolality, focal symptoms subsided.

Estados de hipercoagulabilidad heredados y trombosis venosa cerebral: experiencia en 3 casos / Cerebral venous thrombosis in hereditary hypercoagulability: report of three cases

Hereditary hypercoagulability has been identified as risk factor in approximately 30 percent of cerebral venous thrombosis cases. We report three females with this association. A 38 years old female with a history of deep venous thrombosis of the lower limb, presented with headache, vomiting and a generalized seizure. Magnetic resonance angiography showed a partial thrombosis of the left lateral and superior longitudinal venous sinuses. Coagulation study showed a resistance to activated C protein and factor V Leyden. A 42 years old woman with a history of deep venous thrombosis, presented a right hemiplegia during a hospitalization. Magnetic resonance showed a left lateral hemorrhagic infarction. Magnetic resonance angiography showed an absence of signal in three venous sinuses. Coagulation study showed a protein C deficiency. A 17 years old woman presented a right hemiparesis in the sixth day of puerperium. CAT scan showed a left frontoparietal subcortical venous infarction. Coagulation study showed an antithrombin III deficiency

Caracterización clínica de 450 pacientes con enfermedad cerebrovascular ingresados a un hospital público durante 1997 / Retrospective analysis of 450 patients with cerebrovascular disease admitted to a public hospital during 1997

Background: In Chile, cerebrovascular diseases are the fifth cause of death among men and the third cause among women. Aim: To assess the clinical features and management of patients with cerebrovascular disease admitted to a public hospital during 1997. Patients and methods: A retrospective analysis of clinical records of patients discharged with a diagnosis of cerebrovascular disease. Those records in which there was discordance between the discharge diagnosis and the clinical picture were not considered in the analysis. Results: Of the 563 discharges from the hospital with the diagnosis of cerebrovascular disease, 487 records were located and 450 were considered in the analysis. Fifty four percent of patients were male and ages ranged from 17 to 96 years old...

Psicosis esquizomorfa como forma de presentación de epilepsia parcial compleja / Schizomorphic psychosis as presentation of complex partial epilepsy

Se presenta el cuadro clínico de un hombre de 30 años quien manifiesta características de epilepsia psicomotora y de psicosis epiléptica lúcida y confusa, siendo la psicosis el modo de presentación de la epilepsia parcial compleja. Enfatizamos que las manifestaciones psiquiátricas y conductuales algunas veces constituyen la única expresión de la epilepsia parcial compleja. Se analiza la relación entre epilepsia y psicosis epiléptica, señalando que ambas constituyen la expresión de una lesión en el circuito temporolímbico y la frecuente dificultad en su detección por los métodos electroencefalográficos habituales. Sugerimos que es posible que un ictus prolongado del sistema límbico pueda producir episodios psicóticos, considerados erróneamente interictales en su origen

Brote de carbunco en un sector cordillerano de Nahuelbuta: un caso de enteritis carbuncosa fatal / Outbreak of carbuncle in a mountain area of Nahuelbuta: one case of fatal carbuncular enteritis

Se analiza un brote de carbunco humano aparecido en 1985 en un sector de la Cordillera de Nahuelbuta, comuna de Contulmo, provincia de Arauco, Octava Región. El cuadro afectó a 9 personas en todas las cuales existía el antecedente de ingesta de carne de vacuno muerto a consecuencia de infección por Bacillus anthracis, comprobada por el laboratorio. Las manifestaciones de carbunco cutáneo se presentaron en todos los pacientes, comprobándose clínicamente hemorragia en dos de ellos. En uno la evolución resultó fatal a consecuencia del compromiso gastrointestinal, descrito como muy infrecuente en la literatura revisada. Este caso desafortunado confirma la gravedad y alta letalidad que se atribuye a esta forma de la afección. Se destaca que no existe criterio unificado con respecto al tratamiento de esta enfermedad en el ser humano. No obstante, parece existir buena respuesta a la penicilina sódica en las formas cutáneas, lo que permite aconsejarla como antibiótico de elección en la mayoría de los casos.